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目的探讨肾原发性恶性孤立性纤维性肿瘤(MSFT)的临床病理特征及鉴别诊断,提高对肾MSFT的认识。方法观察1例肾MFST的临床、组织学和免疫组化表型特征,并复习国内外文献。结果患者男性,年龄54岁。体检发现左肾包块。CT示左肾下极12.6 cm×8.7 cm×8.3 cm大小肿块影,肿块内见稍低密度坏死灶,增强扫描病灶呈不均匀强化,可见液化坏死灶,考虑左肾癌。术中见左肾下极大小13 cm×9.5 cm×9 cm卵圆形肿块11个,肿瘤边界清楚,实性、质软,切面灰白、灰黄色。镜下见肿瘤细胞呈密集区和稀疏区交替分布,异型性明显,有出血、坏死,核分裂多见,12个/10HPF。免疫组化示肿瘤细胞vimentin、CD99、CD34、bcl-2弥漫强(+),Ki-67增殖指数20%,EMA、AE1/AE3、CD117、DOG1、desmin、SMA、S-100和HMB45(-)。结论肾MSFT非常罕见,临床及影像学易误诊为肾癌,确诊依靠组织形态学和免疫组化染色,组织学需要与肾肉瘤样肾细胞癌和其他恶性梭形细胞肿瘤鉴别。
Objective To investigate the clinicopathological features and differential diagnosis of primary malignant solitary fibrous tumor of kidney (MSFT) and to improve the understanding of renal MSFT. Methods One case of renal MFST clinical, histological and immunohistochemical phenotypic characteristics, and review of domestic and foreign literature. Results The patient was male, aged 54 years. Physical examination found that the left kidney mass. CT showed a small shadow of 12.6 cm × 8.7 cm × 8.3 cm in the lower left kidney, a slightly lower density necrosis in the mass, and an enhanced enhancement of the lesion. The liquefied necrosis was seen and the left renal cell carcinoma was considered. Intraoperative see the left kidney under the great size of 13 cm × 9.5 cm × 9 cm oval-shaped tumor 11, the tumor clear boundary, solid, soft, cut gray, gray yellow. Microscopic tumor cells showed dense areas and sparse area alternating distribution, atypia obvious bleeding, necrosis, mitotic more common, 12 / 10HPF. Immunohistochemistry showed that vimentin, CD99, CD34, bcl-2 were diffusely strong (+), proliferation index of Ki-67 was 20%, EMA, AE1 / AE3, CD117, DOG1, desmin, SMA, S-100 and HMB45 ). Conclusions Renal MSFT is very rare. Clinical and radiological findings are easily misdiagnosed as renal cell carcinoma. The diagnosis depends on histomorphology and immunohistochemical staining. The histological needs are differentiated from those of sarcomatoid renal cell carcinoma and other malignant spindle cells.